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274 BRITISH MEDICAL JOURNAL VOLUME 283 25 JULY 1981

probable diagnosis of osteomalacia. The extremely low plasma 25-hydroxy 2 Dunnigan MG, McIntosh WB, Sutherland GR, et al. Policy for prevention
vitamin D (25-OHD) concentration confirmed the diagnosis. Calciferol of Asian rickets in Britain: a preliminary assessment of the Glasgow
replacement treatment 3000 units daily was started. Two months later a rickets campaign. Br Med 7 1981 ;282:357-60.
mean unilateral hearing improvement of 12 db was apparent. 3 Weir N. Sensorineural deafness associated with recessive hypophospha-
taemic rickets. J Laryngol Otol 1977;91 :717-22.
4 Elies W, Plester D. Basilar impression-a differential diagnosis of Meniere's
CASE 2
disease. Arch Otolaryngol 1980;106:232-3.
A 49-year-old vagrant Irish ex-labourer was admitted in a state of con- 5 Chakrabarti AK, Johnson SC, Samantray SK, Reddy ER. Osteomalacia,
fusion, dishevelment, and dehydration. One year previously he reported the myopathy and basilar impression. 7 Neurol Sci 1974;23:227-35.
onset of muscular weakness, progressive bilateral deafness, tinnitus, and (Accepted 23 April 1981)
episodic vertigo. Neurological examination showed gross wasting and weak-
ness of the small hand, calf, and deltoid muscles. The tympanic membranes
were normal; there was no spontaneous nystagmus. Audiometry showed a Department of Otolaryngology, The London Hospital, London El
trough-shaped 55 db bilateral recruiting deafness, and caloric testing bilateral 1BB
canal hypofunction. G B BROOKES, FRCS, senior registrar
Biochemical evaluation (table) showed a reduced 25-OHD concentration, A W MORRISON, FRCS, DLO, senior consultant surgeon
which was consistent with osteomalacia, probably due to inadequate dietary
vitamin D intake. Results of cochlear tomography were normal, but skull
radiography showed pronounced basilar impression. The temporal relation
of the auditory and muscular symptoms suggested a direct correlaton. He
discharged himself from hospital before treatment could be started.

Results of preliminary biochemical evaluation and radiology in two patients


Valproic acid in Sydenham's
chorea
Case 1 Case 2
Valproic acid is an effective anticonvulsant that may act by facilitating
Plasma variables the gamma-aminobutyric acid (GABA) neurotransmitter system.'
Venereal disease research laboratory test
Treponemal haemagglutination test L Negative Negative Patients with Huntington's chorea who have low brain concentrations
Fluorescent treponemal antibody of GABA do not respond to treatment with valproic acid,2 3 but one
absorption test
Calcium (corrected) (mmol/l)
f 2-16 2-56 patient with familial paroxysmal choreoathetosis improved when
Inorganic phosphate (mmol/l) 0-68 0 90 taking the drug.4 I present the first report of a patient with Sydenham's
Alkaline phosphatase (U/I) 193 482
Urea (mmol/l) 40 2-7 chorea treated with valproic acid.
Creatinine (,umol/l) 66 55
25-OHD (nmol/l)* <5 3 15-3
Radiology Case report
Skull Normal Pronounced
vertebrobasilar
impression A 19-year-old previously healthy woman developed twitching of the right
Cochlear tomography Demineralisation Normal hand that progressed to all parts of the body over the next two months.
Skeletal survey Normal Normal
Stress or fatigue exacerbated the movements, which disappeared during
sleep. She came to hospital when she began to have slurred speech and
*Measured by competitive protein-binding assay. difficulty in walking. She had no neurological symptoms apart from
Conversion: SI to traditional units-calcium, inorganic phosphate: 1 mmol/l emotional lability noted by her mother. Although she had experienced
2 mEq/l. Urea: 1 mmol/1v6 mg/100 ml. Creatinine: 1 sLmol/l 11 3 ,ug/100 ml. frequent upper respiratory infections, she could not recall any specific illness
25-OHD: 1 nmol/l = 0 4 ng/ml.
before the onset of her movement disorder. A non-pruritic, evanescent, red
rash had been present on her trunk and proximal limbs for two months, and
Comment during the first month of her illness her right shoulder and right thumb
ached. At no time did she have fever, joint swelling, chest pain, or shortness
It is generally accepted that serum abnormalities of calcium, of breath. There was no family history of chorea.
inorganic phosphate, alkaline phosphatase, and 25-hydroxycholecal- On examination the most prominent finding was pronounced choreo-
athetosis of the arms, legs, trunk, face, eyes, and tongue. Muscle tone was
ciferol or 25-OHD may enable early osteomalacia to be diagnosed in the reduced. She had a macular, erythematous, irregular rash on the trunk and
absence of radiological features of demineralisation. In the series of upper arms during the first day in hospital. There was a moderate aortic
Dunnigan et al only 29%/ of children with biochemical evidence of systolic ejection murmur and softer mitral systolic murmur at the apex. She
rickets showed radiological abnormalities.2 Localised bone pain and did not have fever, arthritis, or skin nodules. Antistreptolysin titre was 166
muscle wasting are recognised symptoms of osteomalacia, but deafness Todd units. The following investigations were normal: erythrocyte sedi-
has not been reported as a presenting feature. mentation rate; C-reactive protein concentration; titres of antihyaluronidase,
Deficiency of vitamin D or its metabolic derivatives 25-hydroxy- antinuclear antibody, and anti-DNA; lupus erythematosus test; serum
cholecalciferol and 1,25-hydroxycholecalciferol may result directly protein electrophoresis; throat swab; concentrations of triiodothyronine,
in auditory dysfunction, or exert a secondary effect by disturbances of thyroxine, and electrolytes; cerebrospinal fluid; electroencephalography;
computed tomography; electrocardiography; echocardiography; and chest
calcium metabolism. Calcium ions play an important part in membrane x-ray examination.
permeability. Active transport mechanisms that include ATPase The oral contraceptive pill that she had been taking for one year was
activity, which maintain the differential biochemical integrity of the stopped and she was given diazepam and penicillin. Her chorea continued to
inner ear fluids vital for normal cochlear function, may be calcium worsen until her speech was unintelligible and she had difficulty in swallow-
dependent. In addition, ionised calcium is necessary for normal nerve ing and could not walk unassisted. Valproic acid 250 mg twice daily was
function and deficiency may adversely affect transmission of the nerve started; within six hours the chorea had begun to lessen and after 48 hours
action potentials generated by the cochlea. Retrocochlear deafness in she could walk and swallow normally. Speech also improved but mild
chorea persisted. After four days the valproic acid was stopped. By the next
hereditary vitamin-D-resistant rickets, due to secondary hyperpara- day her chorea and dysarthria had worsened. When the drug was restarted
thyroidism causing osteosclerotic narrowing of the internal auditory again at the same dose three days later speech returned to normal and the
canals, has been described.3 chorea lessened to affect only the arms and abdominal muscles. Chorea
Basilar impression is a bony, vascular, and neural malformation in gradually disappeared over the next month and the drug was stopped.
the craniocervical region that may be associated with deafness and
episodic vertigo.4 The acquired type is rare but may occur secondary to
osteomalacia.5 In case 2 we are not certain whether a direct relation Comment
between the two conditions existed as no other skeletal radiological
abnormalities were present. Finally, demineralisation of the otic When the presenting symptom is chorea, as in this patient,
capsule may lead to secondary degenerative changes in the spiral laboratory tests often do not confirm the diagnosis of rheumatic fever.
ligament, stria vascularis, and cochlear hair cells. Whatever the Other symptoms and signs and the absence of other causes support
mechanism, the threshold improvement in case 1 after treatment the diagnosis of Sydenham's chorea, although the oral contraceptive
suggests a previously unrecognised causal correlation between vitamin pill used by this patient may have influenced the severity of the
D deficiency and cochlear deafness. disease.
The rapid response to low-dose valproic acid and the subsequent
Morrison AW. Diseases of the otic capsule. In: Ballantyne J, Groves J, deterioration when the drug was stopped support the effectiveness of
eds. Scott-Brown's diseases of the ear, nose and throat. Vol 2. 4th ed. the treatment. Valproic acid increases GABA throughout the body,5
London: Butterworths, 1979:465-98. but its effectiveness in chorea is probably centrally mediated. Although
BRITISH MEDICAL JOURNAL VOLUME 283 25 juLy 1981 275

chorea occurs when the function of the corpus striatum is disrupted, Patients with indwelling Tenckhoff catheters, and especially those
the specific abnormality in Sydenham's chorea is unknown. The receiving ambulatory peritoneal dialysis, should be advised of the
response to valproic acid suggests that the defect affects the GABA infective risk of swimming in salt water. When such patients with
neurotransmitter system. Possibly the difference in effectiveness of marine contact develop peritonitis the possibility that the causative
valproic acid between Sydenham's chorea and Huntington's chorea organism may be a marine vibrio, such as V alginolyticus, should be
reflects the degree of severity of the pathologic process or, alternatively, considered.
indicates a difference in the pathogenesis of the two diseases.
lSchmidt U, Chmel H, Cobbs C. Vibrio alginolyticus infections in humans.
I Browne TR. Valproic acid. N Engl7 Med 1980;302:661-6. 7 Clin Microbiol 1979;10:666-8.
2 Perry TL, Hansen S, Kloster M. Huntington's chorea: deficiency of 2 Prociv P. Vibrio
alginolyticus in Western Australia. MedJ7 Aust 1978;2:
gamma-aminobutyric acid in brain. N EnglJ Med 1973;288:337-42. 296.
3Lenman JAR, Ferguson IT, Fleming AM, et al. Sodium valproate in 3 Pien F, Lee K, Higa H. Vibrio alginolyticus infections in Hawaii. Y Clin
chorea. Br Med J' 1976;ii: 1107-8. Microbiol 1977 ;5 :670-2.
Suber DA, Riley TL. Valproic acid and normal CT scan in kinesiogenic Rubin SJ, Tilton RC. Isolation of Vibrio alginolyticus from wound
familial paroxysmal choreoathetosis. Arch Neturol 1980;37:327. infections. 7 Clin Microbiol 1975 ;2 :556-68.
Loscher W, Schmidt D. Increase of human plasma GABA by sodium Ryan WJ. Marine vibrios associated with superficial septic lesions. J Clin
valproate. Epilepsia 1980;21 :611-5. Pathol 1976;29:1014-5.
(Accepted 29 April 1981) (Accepted 29 April 1981)

Oshawa General Hospital, Oshawa, Ontario, Canada Departments of Microbiology and Nephrology, The Royal Melbourne
R S McLACHLAN, MD, FRCP(C), neurologist Hospital, Melbourne, Victoria, Australia 3050
ROD TAYLOR, MB, BS, nephrology resident
MALCOLM McDONALD, FRACP, microbiology registrar
GRAEME RUSS, FRACP, nephrology registrar
MARA CARSON, RT, microbiology technologist
ELIZABETH LUKACZYNSKI, BAPPSC, microbiology technologist
Vibrio alginolyticus peritonitis
associated with ambulatory
peritoneal dialysis
Respiratory and bulbar paralysis
Vibrio alginolyticus is an unusual human pathogen. It is related to V
parahaemolyticus and its natural habitat is the sea. Human infections with relapsing hyperthyroidism
reported to date have been mostly wound and ear infections in
swimmers and others with marine contact. We believe that the follow- Bulbar paresis occurring in patients with hyperthyroidism is a
ing is the first reported case of V alginolyticus peritonitis. well-recognised phenomenon.' 2 Reports have cited only difficulty in
swallowing and aspiration pneumonia as serious complications of the
bulbar weakness. We report a case of hyperthyroid bulbar myopathy
Case report that required artificial ventilation to support life.
A 20-year-old man with end-stage renal failurc, who had been receiving
treatment for six months with continuous ambulatory peritoneal dialysis,
presented with signs and symptoms of peritonitis. Several days previously Case report
he had been scuba-diving off the South Australian coast and had changed his
peritoneal dialysis fluid on the beach without taking adequate precautions A 49-year-old Chinese woman presented with intermittent diplopia and
against infection. difficulty in swallowing of three weeks' duration. Swallowing fluids often
He presented with severe abdominal pain, appreciable fever, and signs of resulted in coughing and spluttering. She noticed that she could no longer
peritonism. Samples of macroscopically turbid peritoneal fluid were taken whistle. One week after the onset of the symptoms ethinyloestradiol
immediately from the Tenckhoff catheter for microscopy and culture. Gram (50 [g) daily had been prescribed. In 1958 she had undergone paidal
staining showed pus cells but no organisms. Treatment was started with thyroidectomy for hyperthyroidism in Hong Kong. This had been repeated
continuous peritoncal lavage with flucloxacillin (50 mg/l) added to the in 1968 because of a recurrence of hyperthyroidism.
dialysis fluid. His symptoms improved but oxidase-positive, Gram-negative Examination showed a fit woman with a resting pulse of 96 beats/min and
bacilli resistant to flucloxacillin (420 colonies/ml) were cultured from the normal blood pressure. There was no thyromegaly or thyroid bruit. Diplopia
first sample of dialysis fluid collected. The treatment was changed to with weakness of the right inferior rectus muscle was present intermittently.
gentamicin 4 mg/l as Pseudomonas was considered to be the likely infecting No exophthalmos or lid lag was noted. Definite bilateral facial weakness,
organism. poor palatal movement, and bilateral tongue weakness were evident. Mild
Further identification was carried out using routine biochemical tests. Tl-e shoulder girdle weakness was present. Tendon reflexes and sensory testing
initial reactions suggested that the organism was possibly a member of the were entirely normal. Routine haematology, electrolyte concentrations, and
Bacillus genus. Since Gram staining showed large Gram-negative rods, liver function tests, including creatine kinase activity, were normal.
however, the organism was resubmitted for appareil proc6d6s d'identification Electromyography and nerve conduction studies were normal, and an
(API) and conventional tests. As the API system indicated that the organism edrophonium test (10 mg) was negative. Barium swallow showed no
was a vibrio ( ?V cholerae) his history was reviewed and marine vibrio peristalsis. Direct laryngoscopy showed no movement of the cords. Thyroid
suspected. Further identification procedures showed the organism to be V function tests showed: thyroxine concentration 237 nmol/l (18-4 yg/100 ml)
alginolyticus, sensitive to aminoglycosides, co-trimoxazole, cephalothin, and (normal 70-140 nmol/l; 5-4-10-9 ,tg/1O0 ml); unbound thyroxine binding
cephalexin but resistant to ampicillin. globulin 95 ,' (85-110 of normal pool); and triiodothyronine 2-8 nmol/l
He made a satisfactory recovery over this period, and after four days (182 ng/100 ml) (normal 1-1-2 6 nmol/l; 72-169 ng/100 ml). A thyroid
cultures of the peritoneal fluid were negative and the gentamicin was pertechnetate scan showed increased uptake in the right and pyramidal lobes.
stopped. He was discharged from hospital taking oral cephalexin and The antithyroid (microsomal) antibody titre was 1/6400 and thyroglobulin
remained in good health. antibody titre 1/320.
Soon after admission to hospital she required endotracheal intubation
and artificial respiration as her vital capacity had fallen below 600 ml, she
Comment could Ino longer swallow, and arterial blood-gas analysis showed oxygen
pressure 8-1 kPa (61 mm Hg), carbon dioxide pressure 7-2 kPa (54 mm Hg),
Peritonitis is the most important complication of ambulatory and pH 7-33. Ventilation was required for six weeks. At the end of this
peritoneal dialysis. The infective organism is usually from the patient's period bulbar and respiratory muscle power had almost recovered. During
own flora and infection occurs after breakdown of the aseptic technique. her admission treatment consisted of carbimazole 20 mg daily and predniso-
In this case the infecting organism, V alginolyticus, was of marine lone 100 mg on alternate days. Thyroid function (biochemically) had
returned to normal two weeks before she could be weaned off the ventilator.
origin. It has a worldwide distribution1 and can be isolated from fish At follow-up three months later she was well with no symptoms.
and crustacea.2 It differs from V parahaemolyticus in that it ferments
sucrose, is Voges-Proskauer positive, will grow in both 8% and 10%
sodium chloride, and swarms on blood agar. An unusual cause of Comment
human infection, V alginolyticus has usually been associated with skin
and ear infections'-5 acquired in a marine setting. To our knowledge In patients with hyperthyroidism weakness may be due to a
V alginolyticus peritonitis has not been reported previously. thyrotoxic myopathy, hypokalaemic periodic paralysis3 (particularly

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